All issues > Volume 41(3); 1998

Case Report

J Korean Pediatr Soc. 1998;41(3):415-419. Published online March 15, 1998.

A Case of Congenital Nephrotic Syndrome due to Diffuse Mesangial Sclerosis

Jung-Jin JJ Yu¹, Dong Kyu DK Jin¹, Hae Il HI Cheong¹, Hyun Soon HS Lee², Yong Y Choi¹

¹Department of Pediatrics, Seoul National University, College of Medicine, Seoul, Korea
²Department of Pathology, Seoul National University, Seoul National University

Correspondence Jung-Jin JJ Yu ,Email: 1

Abstract

Diffuse mesangial sclerosis(DMS) is one of the underlying pathology of congenital and infantile nephrotic syndrome. Infants with DMS develop nephrotic syndrome before 2 years of age and progress to end stage renal disease within 3 years of age. The authors experienced a case of isolated DMS in a 4-month-old male infant who had nephrotic syndrome for 1 month. The diagnosis was confirmed on the basis of clinical, laboratory, pathological and molecular genetic findings. This is the 3rd case report of DMS in our country and the 1st case report of isolated DMS confirmed by molecular genetic study.

Keywords :Infantile nephrotic syndrome, Diffuse mesangial sclerosis, Denys-Drash syndrome, WT1 gene